congenital heart-disease1506
TRANSCRIPT
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Congenital Heart Disease
Thoracic Conference
Frank Nami, M.D.
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The Heart
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Congenial Heart Disease
• Obstructive Congenital Heart Lesions
• Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow
• Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow
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Obstructive Congenital Heart Lesions
• Impede the forward flow of blood and increase ventricular afterloads.
• Pulmonary Stenosis
• Aortic Stenosis
• Coarctation of the Aorta
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Pulmonary Stenosis
• No symptoms in mild or moderately severe lesions.
• Cyanosis and right-sided heart failure in patients with severe lesions.
• High pitched systolic ejection murmur maximal in second left interspace.
• Ejection click often present.
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Pulmonary Stenosis
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Aortic Stenosis
• Valvular Aortic Stenosis
• Subaortic Stenosis
• Supravalvular Aortic Stenosis
• Asymmetric Septal Hypertrophy (Idiopathic Hypertrophic Subaortic Stenosis)
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Valvular Aortic Stenosis
• Most common type, usually asymptomatic in children.
• May cause severe heart failure in infants.
• Prominent left ventricular impulse, narrow pulse pressure.
• Harsh systolic murmur and thrill along left sternal border, systolic ejection click.
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Valvular Aortic Stenosis
• Predominantly in males
• Thickened, fibrotic, malformed aortic leaflets.
• Fused commissures
• Bicuspid aortic valve.
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Valvular Aortic Stenosis
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Coarctation of the Aorta
• Absent or weak femoral pulses.
• Systolic pressure higher in upper extremities than in lower extremities; diastolic pressures are similar.
• Harsh systolic murmur heard in the back.
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Coarctation of the Aorta
• Males twice as frequently as females.
• 98% of all coarctations at segment of aorta adjacent to ductus arteriosus.
• Produced by both an external narrowing and an intraluminal membrane.
• Blood flow to the lower body maintained through collateral vessels.
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Coarctation of the Aorta
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Congenital Heart Lesions that INCREASE Pulmonary Arterial
Blood Flow• Atrial Septal Defect• Complete Atrioventricular Canal• Ventricular Septal Defect• Patent Ductus Arteriosis
• Total Anomalous Pulmonary Venous Connection• Truncus Arteriosus
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Atrial Septal Defect
• Acyanotic; asymptomatic, or dyspnea on exertion.
• Right ventricular lift.
• Fixed, widely split second heart sound.
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Atrial Septal Defect
• Average life expectancy reduced because of right ventricular failure, dysrhythmias, and pulmonary vascular disease.
• Surgical closure is recommended.
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Atrial Septal Defect
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Atrial Septal Defect
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Atrial Septal Defect
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Complete Atrioventricular Canal
• Heart failure common in infancy.
• Cardiomegaly, blowing pansystolic murmur, other variable murmurs.
• Deficiencies of both atrial and ventricular septal cushions and abnormalities of both mitral and tricuspid valves.
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Complete Atrioventricular Canal
• Partial and complete AV canal defects frequently accompany Down’s syndrome.
• Early surgical correction.
• Reconstruction of the AV valves and closure of the septal defects by a single or double patch technique.
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Complete Atrioventricular Canal
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Complete Atrioventricular Canal
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Ventricular Septal Defect
• Asymptomatic if defect is small.
• Heart failure with dyspnea, frequent respiratory infections, and poor growth if defect is large.
• Pansystolic murmur maximal at the left sternal border.
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Ventricular Septal Defect
• Often one component of another more complex congenital heart lesion.
• Heart is enlarged and lung fields are overcirculated.
• Many of the defects will close spontaneously by age 7-8 years.
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Ventricular Septal Defect
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Ventricular Septal Defect
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Patent Ductus Arteriosis
• Murmur usually systolic, sometimes continuous, “machinery”
• Poor feeding, respiratory distress, and frequent respiratory infections in infants with heart failure.
• Physical exam and echocardiography.
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Patent Ductus Arteriosis
• Indomethacin, a prostaglandin E1 inhibitor may close a PDA.
• Surgical treatment after one week, by ligation, clipping, or division.
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Patent Ductus Arteriosis
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Patent Ductus Arteriosis
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Total Anomalous Pulmonary Venous Connection
• Pulmonary veins do not make a direct connection with the left atrium.
• Blood reaches the left atrium only through an atrial septal defect or patent foramen ovale.
• Pulmonary congestion, tachypnea, cardiac failure, and variable cyanosis.
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Total Anomalous Pulmonary Venous Connection
• Diagnosis by cardiac catherization or echocardiography.
• Operative repair in all cases.
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Truncus Arteriosus
• Single large vessel overrides the ventricular septum and distributes all the blood ejected from the heart.
• Large VSD is present.
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Truncus Arteriosus
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Truncus Arteriosus
• Corrective operation with a valved conduit between right ventricle and pulmonary vessels.
• Conduit will need to be changed as child grows but likelihood to develop pulmonary vascular disease is greatly reduced.
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Congenital Heart Lesions that DECREASE Pulmonary Arterial
Blood Flow
• Tetralogy of Fallot
• Transposition of the Great Arteries
• Tricuspid Atresia
• Ebstein’s Anomaly
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Tetralogy of Fallot
(1) Pulmonary stenosis
(2) VSD of the membranous portion
(3) Overriding aorta
(4) Right ventricular hypertrophy due to shunting of blood
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Tetralogy of Fallot
• Addition of an atrial septal defect falls in the category of Pentalogy of Fallot.
• Hypoxic spells and squatting.
• Cyanosis and clubbing.
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Tetralogy of Fallot
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Transposition of the Great Arteries
• Aorta from right ventricle, pulmonary artery from left ventricle.
• Cyanosis from birth, hypoxic spells sometimes present.
• Heart failure often present.
• Cardiac enlargement and diminished pulmonary artery segment on x-ray.
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Transposition of the Great Arteries
• Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA.
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Transposition of the Great Arteries
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Transposition of the Great Arteries
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Tricuspid Atresia
• Tricuspid valve is completely absent in about 2% of newborns with congenital heart disease.
• Blood flows from right atrium to left atrium through foramen ovale.
• Early cyanosis.
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Tricuspid Atresia
• Repair consists of shunt from right atrium to pulmonary artery or rudimentary right ventricle (Fontan procedure).
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Ebstein’s Anomaly
• Septal and posterior leaflets of the tricuspid valve are small and deformed, usually displaced toward the right ventricular apex.
• Most patients have an associated ASD or patent foramen.
• Cyanosis and arrhythmias in infancy are common.
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Ebstein’s Anomaly
• Right heart failure in half of patients.
• Operative repair with tricuspid valve replacement.
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Congenital Heart Disease
• The end, thank you.